Late Gadolinium Enhancement Score Research Articles

The predictive role of right ventricular late gadolinium enhancement in patients with tetralogy of Fallot undergoing pulmonary valve replacement

BackgroundOur purpose was to evaluate the correlations between right ventricular (RV) late gadolinium enhancement (LGE) at cardiac magnetic resonance (CMR) in patients with tetralogy of Fallot (ToF) scheduled for pulmonary valve replacement (PVR) and post-PVR functional data.MethodsWe retrospectively reviewed ToF patients scheduled for PVR who underwent two CMR examinations at our institution, one before the procedure (CMR-0), including contrast-enhanced sequences, and one after the procedure (CMR-1). Functional left and RV data were obtained by segmenting short-axis stacks on both CMR examinations, and normalised variations were calculated by dividing differences between CMR-1 and CMR-0 by the intercurring time interval, whereas the RV scar burden was assessed on CMR-0 LGE sequences both semiquantitatively and quantitatively. Data were reported as median and interquartile range, differences were appraised with the Mann–Whitney U test, while correlations were assessed with Spearman’s ρ.ResultsFifteen patients with a median age of 25 years (16–29), including 9 (60%) males, with a median time interval between CMR-0 and CMR-1 of 17 months (12–23), were retrospectively reviewed. The semiquantitative LGE score at CMR-0 was 7 (6–9), and LGE volume was 4.49 mL (3.70–5.78), covering 5.63% (4.92–7.00) of the RV. RV LGE score showed a moderate positive correlation with the normalised variation of RV stroke volume (ρ = 0.662, p = 0.007) and a borderline moderate positive correlation with the normalised variation of RV end-diastolic indexed volume (ρ = 0.513, p = 0.050).ConclusionsThe assessment of RV LGE before PVR may provide insights on post-PVR functional data, potentially facilitating a patient-tailored treatment pathway.

Abstract 12059: Speckle-Tracking Echocardiography Derived Global Circumferential Strain Predicts Adverse Outcomes in Cardiac Sarcoidosis With Preserved Ejection Fraction

Introduction: Risk stratification in cardiac sarcoidosis (CS) remains challenging, especially in those with preserved left ventricular ejection fraction (LVEF). Myocardial strain parameters are more sensitive than LVEF for detecting early sub-clinical myocardial dysfunction, but their prognostic value in CS is not known. Methods: We investigated consecutive newly diagnosed CS patients between 2015 and 2018. All patients underwent 2D echocardiography including global longitudinal strain (GLS) and global circumferential strain (GCS) measurement, cardiac magnetic resonance (CMR) for late gadolinium enhancement (LGE) and FDG-PET imaging to derive the maximum standard uptake value (SUVmax). The LGE score was quantified as the number of myocardial segments with LGE. Major adverse cardiac events (MACE) were all-cause death, sustained ventricular arrhythmia (VA), device implantation and new onset heart failure (HF) admission. Results: A total of 120 patients were included with age of 55±12 years; 87% were Caucasian, 43% had hypertension and 13% had diabetes. Mean LVEF was 55±12.3%, GLS was -13.4±4.2%, GCS was -16.0±5.6% and regional wall motion abnormalities (RWMA) occurred in 36% of cases. During median follow-up of 4.3 years, 43 patients experienced MACE consisting of 15 deaths, 19 VAs, 7 devices and 2 HF admissions. In multivariate analysis syncope, ethnicity, echo-derived left atrial volume and RWMA were independent predictors of MACE along with CMR-derived right ventricular EF and LGE score. Although GLS and GCS were lower in those with MACE, neither had independent prognostic value. SUVmax was also not predictive of events. In a subgroup analysis of the 86 patients with LVEF ≥50%, 20 patients experienced MACE. Multivariate analysis showed non-Caucasian ethnicity (HR 3.18 [95% CI 1.02-9.88]; P = 0.04), RWMA (HR 3.76 [95% CI 1.29-10.98]; P = 0.02), LGE-score (HR 1.26 95% CI 1.05-1.51]; P = 0.01 and GCS (HR 1.18 [95% CI 1.04-1.36]; P = 0.01) were independent predictors of MACE. A GCS value above the optimal cut-off of -16.4% was associated with a worse outcome (log-rank P = 0.005). Conclusion Ethnicity, RWMA and LGE-score are robust markers of outcome in newly diagnosed CS. Reduced GCS provides additional prognostic information in those with preserved LVEF.

Pericardial Involvement in ST-Segment Elevation Myocardial Infarction as Detected by Cardiac MRI.

BackgroundPost myocardial infarction pericarditis is considered relatively rare in the current reperfusion era. The true incidence of pericardial involvement may be underestimated since the diagnosis is usually based on clinical and echocardiographic parameters.ObjectivesThis study aims to document the incidence, extent, and prognostic implication of pericardial involvement in ST-segment elevation myocardial infarction (PISTEMI) using cardiac MRI (CMR).MethodsOne hundred and eighty-seven consecutive ST-segment elevation myocardial infarction patients underwent CMR on day 5 ± 1 following admission, including steady-state free precession (SSFP) and late Gadolinium enhancement (LGE) sequences. Late Gadolinium enhancement and microvascular obstruction (MVO) were quantified as a percentage of left ventricular (LV) mass. Late Gadolinium enhancement was graded for transmurality according to the 17 AHA left ventricle (LV) segment model (LGE score). Late pericardial enhancement (LPE), the CMR evidence of pericardial involvement, was defined as enhanced pericardium in the LGE series and was retrospectively recorded as present or absent according to the 17 AHA segments. Late pericardial enhancement was evaluated adjacent to the LV, the right ventricle, and both atria. Clinical, laboratory, angiographic, and echocardiographic data were collected. Clinical follow-up for major adverse cardiac events (MACE) was documented and correlated with CMR indices, including LGE, MVO, and LPE.ResultsLate pericardial enhancement (LPE+) was documented in 77.5% of the study cohort. A strong association was found between LPE and the degree and extent of myocardial injury (LGE, MVO). Both LGE and MVO were significantly correlated with increased MACE on follow-up. On the contrary, LPE presence, either adjacent to the LV or the other cardiac chambers, was associated with a lower MACE rate in a median of 3 years of follow-up HR 0.39, 95% CI (0.21–0.7), p = 0.002, and HR 0.48, 95% CI (0.26–0.9), p = 0.02, respectively.ConclusionsPrognostic implication of pericardial involvement in ST-segment elevation myocardial infarction was documented by CMR in 77.5% of our STEMI cohort. Late pericardial enhancement presence correlated significantly with the extent and severity of the myocardial damage. Unexpectedly, it was associated with a considerably lower MACE rate in the follow-up period.

Predictive Value of Echocardiographic Strain for Myocardial Fibrosis and Adverse Outcomes in Autoimmune Diseases

BackgroundMyocardial fibrosis is an important pathophysiologic mechanism of cardiac involvement that leads to increased mortality in patients with autoimmune diseases (AIDs). The aim of this study was to evaluate the association between myocardial strain from speckle-tracking echocardiography (STE) and fibrosis on cardiovascular magnetic resonance (CMR) and to further explore their prognostic implications in patients with AIDs.MethodsWe prospectively included 102 AIDs patients with clinically suspected cardiac involvement and 102 age- and sex-matched healthy individuals. Patients underwent CMR for evaluation of myocardial fibrosis by late gadolinium enhancement (LGE) and T1 mapping. A semiquantitative evaluation based on the extent of LGE was used to calculate the total (tLGEs) and segmental (sLGEs) LGE score. Global longitudinal strain (GLS) was evaluated by STE in all subjects. All patients were regularly followed up every 6 months. The primary endpoint was the composite incidence of all-cause death and cardiovascular hospitalization.ResultsCompared to healthy controls, AIDs patients had impaired GLS (−17.9 ± 5.1% vs. −21.2 ± 2.5%, p < 0.001). LGE was detected in 70% of patients. Patients with LGE presented worse GLS (−17.1 ± 5.3% vs. −19.6 ± 4.1%, p = 0.018) than those without LGE. On multivariate logistic analysis, GLS ≥ −15% was an independent predictor of LGE presence (OR = 4.98, 95%CI 1.35–18.33, p = 0.016). Moreover, a marked and stepwise impairment of segmental longitudinal strain (−19.3 ± 6.6 vs. −14.9 ± 6.5 vs. −8.9 ± 6.3, p < 0.001) was observed as sLGEs increased. During a median follow-up time of 25 months, 6 patients died, and 14 patients were hospitalized for cardiovascular reasons. Both GLS ≥ −15% (HR 3.56, 95%CI 1.28–9.86, p = 0.015) and tLGEs ≥ 6 (HR 4.13, 95%CI 1.43–11.92, p = 0.009) were independently associated with the primary endpoint.ConclusionsIn AIDs patients, impaired myocardial strain on STE could reflect the presence and extent of myocardial fibrosis and provide incremental prognostic value in addition to LGE in the prediction of adverse outcomes.

Predictive factors of outcome in patients with Tetralogy of Fallot (TOF) after pulmonary valve replacement: preliminary data of our Centre experience (2010-2020)

Abstract Funding Acknowledgements Type of funding sources: None. Background TOF patients undergo multiple surgical procedures during their life. The improvement in surgical techniques and patient’s clinical management led to longer life expectancy, creating an adult TOF population that experiences specific adverse events, such as atrial/ventricular arrythmias, heart failure and sudden cardiac death. In selected patients, pulmonary valve replacement (PVR) is mandatory to reduce right ventricular (RV) volume; therefore, careful patients’ selection but also correct timing of PVR are crucial. Purpose Current guidelines are based on pre-PVR threshold values of indexed RV end-diastolic volume (RVEDVi) calculated with cardiac magnetic resonance (CMR), to achieve normalization of RV size after valve implantation, but recent studies demonstrated that post-PVR RV dilatation is not so tightly connected with adverse clinical outcomes. Therefore, our aim is to identify new possible predictive factors of post-PVR outcome to provide tailored PVR on the correct cohort of patients, at the correct time. Methods: The inclusion criteria of this retrospective and partially prospective cohort study were i) repaired TOF; ii) available CMR data pre- and post-PVR; iii) PVR performed between 2010 and 2020; iv) clinical and ECG available pre- and post-PVR; v) complete echocardiographic examination pre- and post-PVR. Multivariate analysis was applied. Primary outcomes were considered death, resuscitated death, atrial/ventricular arrhythmias. NYHA class was considered to evaluate patients’ clinical status. CMR Late Gadolinium Enhancement (LGE) score was calculated pre- and post-PVR. Results 22 patients were selected (12 males, median age ± SD 28,25 ± 11,2 years). After PVR, the only primary outcome observed was sustained ventricular tachycardia in 4 patients. Multivariate analysis showed that patients with later PVR, lower Fractional Area Change (FAC) at pre-PVR echocardiogram and higher LGE score post-PVR (LGE &amp;gt; 3) showed higher post-PVR RVEDVi. On the contrary, patients with class I NYHA post-PVR showed lower RVEDVi. Conclusion Our study demonstrates that lower age at PVR and careful patient’s selection, guided by echocardiographic and CMR findings, lead to greater benefit in terms of good volumetric recovery of the RV and better performance status post-PVR (NYHA class I) in TOF patients after PVR.

Abstract 9417: Prognostic Value and Predictors of Right Ventricular Dysfunction by Magnetic Resonance Imaging in Non-Ischemic Cardiomyopathy

Background: Challenges remain in our understanding of implications of right ventricle (RV) dysfunction in heart failure. Cardiac magnetic resonance imaging (CMR) is the reference standard for RV quantification, but has seldomly been studied for this purpose in cardiomyopathy patients. We evaluated the predictors and outcomes of reduced CMR-derived right ventricle function in non-ischemic cardiomyopathy (NICM). Methods: Adult NICM patients undergoing CMR quantifying the RV during 2002-2017 were retrospectively studied. Multivariable linear and Cox proportional hazards regression were used to identify factors associated with reduced RV ejection fraction (RVEF) and adverse outcomes. The primary endpoint was all-cause mortality, heart transplant and/or left ventricular assist device implantation. Results: Amongst 624 NICM patients, mean age was 52.7±15.9 years, 253 (40.5%) were female, and RVEF was 43±13%. Factors independently associated with RVEF (beta-coefficients, P-values) were male (-3.26, P&lt;0.001), New York Heart Association Class (-1.78, P=0.003), left ventricular ejection fraction (0.39, P&lt;0.001), right ventricular end-diastolic volume indexed (-0.15, P&lt;0.001), and mitral regurgitant fraction (MR-RF) (-0.18, P&lt;0.001). Reduced RVEF was independently associated with the primary endpoint (occurring in 113 (18.1%) patients), hazards ratio 0.81 (95% confidence interval 0.74-0.89), with other predictors being age, diabetes, late gadolinium enhancement score and MR-RF(Table). Prognostic thresholds for clinically significant and severe RVEF were determined to be RVEF≤43% and ≤31% respectively. Conclusion: Reduced RVEF was prevalent in NICM patients, and was independently associated with worse prognosis in NICM. Important clinical and CMR factors associated with lower RVEF were identified, as well as the optimal prognostic RVEF thresholds.

18F-sodium fluoride PET/MRI myocardial imaging in patients with suspected cardiac amyloidosis

BackgroundWe evaluated the diagnostic performance of 18F-NaF PET/MRI in patients with suspected cardiac amyloidosis (CA). MethodsTwenty-seven consecutive patients underwent myocardial PET 1 hour after injection of 4 MBq/kg 18F-NaF with simultaneous MRI including cine-MRI, T1 and T2 mapping, first-pass and late gadolinium enhancement (LGE). 18F-NaF uptake was measured visually and semi-quantitatively by calculating myocardium-to-blood pool (M/B) ratios. CA was confirmed histologically. ResultsTransthyretin (TTR)-CA was diagnosed in 16 patients, light-chain (AL)-CA in 7, and no-CA in 4. Visual interpretation of 18F-NaF images revealed a relative increase in myocardial uptake in only 3 patients, all with TTR CA, and a relative decrease in 13, including 7 AL CA, 3 no-CA, and 3 TTR CA. M/B ratios were significantly higher in TTR CA (1.00 ± 0.12) than in AL CA (0.81 ± 0.06, P = 0.001) or in no-CA (0.73 ± 0.16, P = 0.006). The optimal M/B cut-off to distinguish TTR CA from AL CA was ≥ 0.90 (Fischer, P = 0.0005). By comparison, classification of patients using 99mTc-HMDP heart-to-mediastinum ratios with the previously published cut-off ≥ 1.21 reached higher significance (P < 0.0001). Among MRI parameters, myocardial T1, LGE score, and extracellular volume were higher in CA than in no-CA patients, 1409 ± 76 vs 1278 ± 35 ms (P = 0.004), 10.35 ± 5.30 vs 3.50 ± 3.42 (P = 0.03), and 46 ± 10 vs 33 ± 8 % (P = 0.01), respectively. Conclusion18F-NaF PET/MRI shows good diagnostic performance when semi-quantification is used. However, contrast is low and visual interpretation may be challenging in routine. PET/MRI could constitute a one-stop-shop evaluation of amyloid load and cardiac function in patients needing rapid work-up.

Radial global strain by cardiac magnetic resonance imaging predicts arrhythmic outcomes in hypertrophic cardiomyopathy

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Hypertrophic cardiomyopathy (HCM) is a relatively prevalent condition associated with arrhythmic events and sudden cardiac death. Several tools are currently available to identify which HCM patients are at risk of developing these events. Purpose We aimed to evaluate the association of Tissue Tracking strain analysis by cardiac magnetic resonance (CMR) and the development of arrhythmic events in patients with HCM. Methods We prospectively analyzed 136 consecutive patients with HCM diagnosis (established according to current clinical practice guidelines) from January 2006 to October 2017. Every routine 24 hours ECG-monitoring test was registered and looked for sustained or non-sustained ventricular tachycardia (any VT). CMR studies were performed following our predefined CMR protocol for HCM with 1.5T magnets. Cine images were obtained with standard, retrospectively gated, steady-state free-precession (SSFP) sequences in 2, 3 and 4 chambers views and in 10–15 contiguous short-axis slices covering the ventricles from the base to the apex, with breath holding. The strain evaluation was performed by a commercially available Tissue Tracking analysis software, manually defining the endocardial border in short axis, 4, 3 and 2 chambers views and, after verifying adequate identification of the different structures, running the strain analysis (Figure 1, displaying myocardium identification by the strain analysis software). Results Mean follow-up was 49 ± 45 months. Mean age was 61 ± 15 years old (p for the comparation between the group with arrhythmic and the group without arrhythmic events 0.212) and 31% of patients were women (p 0.420). Mean ejection fraction was 69 ± 9.21% (p 0.223) and mean HCM-SCD (hypertrophic cardiomyopathy sudden cardiac death) risk score was 2.20 ± 1.34 (p &amp;lt;0.001). Median percentage of total myocardium showing late gadolinium enhancement (LGE) was 0.61 (interquartile range 2.9; p 0.170). Mean global radial strain (GRS) was 26.23 ± 8.78% (p &amp;lt;0.001). 21 VT episodes were recorded during follow-up. GRS showed an area under de ROC curve of 0.75 predicting VT during follow-up, selecting the value of 27% as the best sensitivity/specificity cutoff point. Statistically significant differences were not found when analyzing global circumferential strain (GCS) and global longitudinal strain (GLS) as VT predictors after adjusting for possible confusion factors (GRS, GCS and GLS distributions depicted in Figure 2). A binary GRS ≥27%/&amp;lt;27% variable was included in a logistic regression model adjusted by age, percent of total myocardium mass showing LGE and HCM-SCD risk score. Significantly more arrhythmic events were found to occur in patients with a GRS &amp;lt;27% (OR 7.33; 95% confidence interval 1.07 – 50.41; p 0.043) after adjusting by age, percent of total myocardium mass showing LGE, and HCM-SCD risk score Conclusions A GRS value of &amp;lt;27% on CMR appears to be a good predictor of worse arrhythmic prognosis in patients with HCM.

Longitudinal changes in extent of late gadolinium enhancement in repaired Tetralogy of Fallot: a retrospective analysis of serial CMRs

BackgroundRight ventricular (RV) late gadolinium enhancement (LGE) occurs due to surgical scarring and RV remodeling, and has been shown to be associated with clinical outcomes in Tetralogy of Fallot (TOF). However, it is not known if cardiovascular magnetic resonance (CMR) LGE extent progresses over time, and therefore, it is not known if serial reassessment of LGE is necessary. We determined the rate of progression in the extent of RV LGE on serial CMR examinations in repaired TOF.MethodsRetrospective review of 127 patients after TOF repair (49% male, median age at first CMR 18.9 years (Interquartile range (IQR) 13.3,27.0) who had at least two CMRs (median follow-up duration of 4.0 years (IQR 2.1,5.9)) was performed. 84/127 patients had no interventions between serial CMRs (Group 1) while 43/127 patients had transcatheter or surgical intervention between CMRs (Group 2). The extent of RV LGE was assessed using 2 methods: a semiquantitative RV LGE score and a quantitative RV LGE extent expressed as % of RV mass. Mixed effects linear regression modeling to estimate changes in LGE over time.ResultsRV LGE was present in all patients on the first CMR. % RV LGE extent and LGE score did not increase over time in either patient group. The mean 5 year rates of change were small and negative for both % RV LGE extent [− 2.3 (95% CI − 2.9, − 1.8, p < 0.001) in Group 1, and − 1.9 (95% CI − 3.2, − 0.7, p = 0.004) in Group 2], and RV LGE score [− 0.9 (95% CI − 1.1, − 0.6, p < 0.001) in Group 1, and − 0.5 (95% CI − 1.1, − 0.0, p = 0.047) in Group 2].ConclusionsIn this serial CMR evaluation of children and adults with repaired TOF, no significant progression in the extent of RV LGE was seen on intermediate term follow-up. Given recent concerns regarding the safety of gadolinium-based contrast agents, frequent assessment of LGE may not be necessary in follow-up.

Fast Strain-Encoded Cardiac Magnetic Resonance for Diagnostic Classification and Risk Stratification of Heart Failure Patients

ObjectivesThe purpose of this study was to compare the ability of fast-strain encoded magnetic resonance (fast-SENC) cardiac magnetic resonance (CMR) to classify and risk stratify all-comer patients with different stages of chronic heart failure (Stages of heart failure A to D) based on American College of Cardiology/American Heart Association guidelines with standard clinical and CMR imaging data. BackgroundHeart failure is a major cause of morbidity and mortality, resulting in millions of deaths and hospitalizations annually. MethodsThe study population consisted of 1,169 consecutive patients between September 2017 and February 2019 who underwent CMR for clinical reasons, and 61 healthy volunteers. In addition, clinical follow-up was performed in Stages A and B patients after 1.9 ± 0.4 years. Wall motion score and late gadolinium enhancement score indexes, left ventricular (LV) ejection fraction, and global circumferential and longitudinal strain based on fast-SENC acquisitions, were calculated in all subjects. The percentage of myocardial segments with strain ≤−17% (% normal myocardium) was determined in all subjects. ResultsLV ejection fraction, global circumferential and longitudinal strain, and % normal myocardium significantly decreased with increasing heart failure stages (p < 0.001 for all by analysis of variance). By multivariable analysis, % normal myocardium remained an independent predictor of heart failure stages, exhibiting closer association than LV ejection fraction (rpartial = 0.76 vs. rpartial = 0.30; p < 0.001). Importantly, 149 of 399 (37%) with Stage A were reclassified to Stage B, that is, as having subclinical LV dysfunction based on % normal myocardium <80%. Such patients exhibited significantly higher rates of all-cause mortality and hospital stay due to heart failure during follow-up, compared with patients with % normal myocardium ≥80% (chi-square = 6.9; p = 0.03). ConclusionsThe % normal myocardium, determined by fast-SENC, enables improved identification of asymptomatic patients with subclinical LV dysfunction compared with LV ejection fraction and risk stratification of patients with so far asymptomatic heart failure. The identification of such presumably healthy patients at high risk for heart failure-related outcomes may bear important medical implications.

The Association between Left Ventricular Diastolic Dysfunction and Myocardial Scar and Their Collective Impact on All-Cause Mortality

Left ventricular diastolic dysfunction (DD) and myocardial scar are important contributors to cardiovascular morbidity and mortality. The aim of this study was to test the hypothesis that myocardial scar is a substrate of DD and that collectively they contribute to increased risk for all-cause mortality. The study included 607 consecutive patients who underwent echocardiography and cardiac magnetic resonance imaging within 1week. DD was assessed by echocardiography according to the contemporary guidelines and myocardial scar by late gadolinium enhancement (LGE). All-cause mortality was the main outcome. Scar prevalence was significantly higher comparing no DD with mild and advanced DD (25%, 61%, and 80%, respectively; P<.001), and scar burden was greater by semiquantitative LGE scores (1.4±3.4, 4.8±6.3, and 6.8±8.8, respectively; P<.001). After 10 years of follow-up, 50 patients (8.2%) had died. The mortality incidence was significantly higher comparing no DD with mild and advanced DD (2.5, 17.3, and 55.7 deaths per 1,000 person-years, respectively; P<.001). It was also higher in patients with higher LGE scores (4.3, 22.7, and 35.2 deaths per 1,000 person-years in groups with LGE scores of 0, 1-6, and ≥7, respectively; P<.001). The risk for death was higher among patients with both DD and scar. The additive interaction of DD and scar for mortality was significant (P<.001). In the multivariate Cox proportional-hazards analysis, DD and scar were associated with mortality (P<.001). There is a strong link between the echocardiographic findings of DD and the morphologic changes of myocardial tissue. DD assessed according to contemporary guidelines effectively differentiates mortality risk. The additive interaction of DD and scar on mortality risk highlights the pivotal role of myocardial tissue characterization in patients with DD.

2395LGE CMR predicts sudden death and VT in adults with repaired tetralogy of Fallot - a prospective study with 3500 patient follow up years

Abstract Background Adults with repaired tetralogy of Fallot (rtoF) are at risk of ventricular arrhythmia and sudden cardiac death (SCD). Cross-sectional data suggest association of late gadolinium enhancement (LGE) cardiovascular magnetic resonance imaging (CMR) with adverse clinical risk factors Purpose We sought to determine prognosis related to LGE CMR. Methods In this prospective cohort study the primary composite outcome comprised the first of cardiovascular death (SCD or heart failure-related), aborted SCD (successfully resuscitated cardiac arrest or appropriate AICD shock for ventricular fibrillation), and clinical sustained ventricular tachycardia (VT&gt;30 seconds duration). Results In 531 rtoF patients (median age 32; 23–42, 296 (56%) male, NYHA≥II 17%) followed up after LGE CMR for median 5 (1.7–8.9) years, there were 39 primary composite outcomes: 10 SCD, 11 heart failure related deaths (2 perioperative RV failure), 2 aborted SCD and 16 clinical sustained VT events. At study end, there were 28 ventricular arrhythmic events in 28 rtoF patients (10 SCD, 16 clinical sustained VT, 2 aborted VF) that were significantly predicted by RV LGE extent (HR 1.45 CI: 1.3–1.6; P&lt;0.001). Univariable predictors of the primary outcome were RV LGE score; HR: 1.44 (1.31–1.57; p&lt;0.001), (Figure) together with older age; HR: 1.05 (1.02–1.07; P&lt;0.001), late repair; HR: 1.04 (1.02–1.07; p&lt;0.001), lower RV ejection fraction; HR: 0.92 (0.89–0.95; p&lt;0.001), larger RVOT akinetic length; HR: 1.04 (1.02–1.06; p&lt;0.001) larger right atrial area; HR: 1.2 (1.12–1.29; p&lt;0.001); higher BNP levels; HR: 1.01 (1–1.02; p&lt;0.001), lower peak VO2; HR: 0.89 (0.83–0.96; p=0.001), prior atrial arrhythmia; HR: 5.3 (2.8–10.07; p&lt;0.001), and non-sustained VT; HR: 4.1 (2.1–7.7; p&lt;0.001). Inducible VT did not predict the primary outcome; HR: 2.1 (0.57–8; p=0.25) In multivariable analysis both RV LGE score and indexed right atrial area (RAAi) only, remained predictive of the primary outcome (HR 1.29 CI: 1.12–1.49; p&lt;0.001 and HR 1.1 CI: 1.02–1.12; p=0.01, respectively). Patients could accordingly be stratified such that supramedian RV LGE score (≥5) and RAAi ≥16cm2/m2 had 5-year event free survival 84% vs 94% for supramedian RV LGE score (≥5) and RAAi &lt;16cm2/m2 or 98% for inframedian RV LGE score with RAAI&lt;16cm2/m2. Figure. Conclusions For every unit increase in CMR defined RV fibrosis score there is a 44% increased risk of sudden cardiac death and VT. LGE CMR and maximal right atrial area should therefore be incorporated into risk stratification for sudden death in adults with rTOF. Acknowledgement/Funding British heart foundation

High Signal Intensity on T2-Weighted Cardiovascular Magnetic Resonance Imaging Predicts Life-Threatening Arrhythmic Events in Hypertrophic Cardiomyopathy Patients.

The prognostic value of high signal intensity on T2-weighted cardiovascular magnetic resonance imaging (T2 high signal) in hypertrophic cardiomyopathy (HCM) patients in a single-center cohort was investigated.Methods and Results:A total of 237 HCM patients (median age, 62 years; 143 male) underwent T2-weighted, cine and late gadolinium enhancement (LGE) imaging, and were followed (median duration, 3.4 years) for life-threatening arrhythmic events. The clinical and magnetic resonance imaging characteristics were extracted, and predictors of life-threatening arrhythmic events were assessed on multivariate analysis. LGE was present in 180 patients (75.9%). Median LGE score was 3 in a left ventricle 17-segment model. T2 high signal was present in 49 patients (20.7%). The annual events rate was significantly higher in patients with extensive LGE (score ≥4) than in those without (3.0%/year vs. 0.5%/year, P=0.011). On multivariate analysis, extensive LGE (hazard ratio, 5.650; 95% CI: 1.263-25.000, P=0.024) as an independent predictor for life-threatening arrhythmic events. In patients with extensive LGE, the annual events rate was significantly higher in patients with T2 high signal than in those without (5.8%/year vs. 0.9%/year, P=0.008). Extensive LGE was an independent predictor of life-threatening arrhythmic events in HCM patients. Furthermore, T2 high signal is useful for the risk stratification of serious arrhythmic events in patients with extensive LGE.

Stress Perfusion CMR in Patients With Known and Suspected CAD: Prognostic Value and Optimal Ischemic Threshold for Revascularization

ObjectivesThis study sought to determine the ischemia threshold and additional prognostic factors that identify patients for safe deferral from revascularizations in a large cohort of all-comer patients with known or suspected coronary artery disease (CAD). BackgroundStress-perfusion cardiac magnetic resonance (CMR) is increasingly used in daily practice for ischemia detection. However, there is insufficient evidence about the ischemia burden that identifies patients who benefit from revascularization versus those with a good prognosis who receive drugs only. MethodsAll patients with known or suspected CAD referred to stress-perfusion CMR for myocardial ischemia assessment were prospectively enrolled. The CMR examination included standard functional adenosine stress first-pass perfusion (gadobutrol 0.1 mmol/kg Gadovist, Bayer AG, Zurich, Switzerland) and late gadolinium enhancement (LGE) acquisitions. Presence of ischemia and ischemia burden (number of ischemic segments on a 16-segment model), and of scar and scar burden (number and transmurality of scar segments in a 17-segment model) were assessed. The primary endpoint was a composite of cardiac death, nonfatal myocardial infarction (MI), and late coronary revascularization (>90 days post-CMR); the secondary endpoint was a composite of cardiac death and nonfatal MI. ResultsDuring a follow-up of 2.5 ± 1.0 years, 86 and 32 of 1,024 patients (1,103 screened patients) experienced the primary and secondary endpoints, respectively. On Kaplan-Meier curves for the primary and secondary endpoints, patients without ischemia had excellent outcomes that did not differ from patients with <1.5 ischemic segments. In multivariate Cox regression analyses of the entire population and of the subgroups, ischemia burden (threshold: ≥1.5 ischemic segments) was consistently the strongest predictor of the primary and secondary endpoints with hazard ratios (HRs) of 7.42 to 8.72 (p < 0.001), whereas age (≥67 years), left ventricular ejection fraction (≤40%), and scar burden (LGE score ≥0.03) contributed significantly, but to a lesser extent, in all models with HRs of 2.01 to 3.48, 1.75 to 1.96, and 1.66 to 1.76, respectively. ConclusionsIn a large all-comer patient cohort with known and suspected CAD, an ischemia burden of ≥1.5 ischemic segments on stress-perfusion CMR was the strongest predictor of the primary and secondary endpoints. Patients with zero or 1 ischemic segment can be safely deferred from revascularizations.

Temporal change of myocardial tissue character is associated with left ventricular reverse remodeling in patients with dilated cardiomyopathy: A cardiovascular magnetic resonance study

BackgroundPrognostic significance of temporal change in myocardial tissue characterization by cardiovascular magnetic resonance (CMR) has not been elucidated in patients with non-ischemic dilated cardiomyopathy (DCM). Methods and resultsSixty-eight patients with newly-diagnosed DCM who underwent CMR including late gadolinium enhancement (LGE) both at baseline and during follow-up period were enrolled. LGE score was defined by a signal intensity of ≥5 standard deviations above the remote reference myocardium mean. Left ventricular reverse remodeling (LVRR) defined as a LV ejection fraction increase of ≥10% and a decrease in indexed LV end-diastolic diameter of ≥10% compared to those at baseline was detected in 38% of the patients. There was no significant difference in LGE score between baseline and follow-up (5.8% vs. 7.3%; p=0.38). The change in LGE area (delta-LGE) was significantly lower in patients with LVRR than those without (−0.5%±3.4% vs. 3.0±7.4%; p=0.02). On the other hand, T2 ratio during the follow-up significantly reduced (1.95±0.48 vs. 1.67±0.56; p<0.01); however, there was no significant difference in the change in T2 ratio between patients with LVRR and those without (−0.29±0.73 vs. −0.27±0.66; p=0.88). Multivariate logistic analysis indicated that baseline LGE score [odds ratio; 0.78; 95% confidence interval (CI) 0.66 to 0.90; p<0.01] together with delta-LGE (odds ratio; 0.77; 95% CI 0.61 to 0.92; p=0.01) were independently associated with subsequent LVRR (p<0.01). ConclusionsThe temporal change of LGE-CMR score during the clinical course was significantly correlated with following LVRR.

Regional myocardial damage and active inflammation in patients with cardiac sarcoidosis detected by non-invasive multi-modal imaging.

Cardiac sarcoidosis (CS) can be diagnosed using 18F-FDG-PET/CT (PET), cardiovascular magnetic resonance (CMR), and 123I-BMIPP/201TlCl dual myocardial SPECT. This study aims to clarify the relationships among the three modalities with respect to CS. We evaluated 16 patients (male n=11; age 55±13years) with confirmed CS who underwent PET, CMR, and dual SPECT with gated SPECT before starting steroid therapy. The left ventricular myocardium was divided into 17 segments to obtain SUVmax for PET images, defect scores from 0 to 4 (0 normal; 4 absent), and mismatch scores for dual SPECT (BMDS, TLDS, and MS) images and late gadolinium enhancement (LGE) scores (0 none; 1 partly positive; 2 homogeneous) on CMR images. Summed BMDS, TLDS, and MS were 18.6±12.6, 12.9±10.9, and 5.7±3.1, respectively. The segmental BMDS and TLDS scores became significantly higher as the LGE scores increased. The MS scores were significantly higher in areas of LGE with a score of 1 than 0 (both, p<0.001), but did not significantly differ between areas with LGE scores of 1 and 2. The SUVmax was significantly higher in LGE areas with a score of 1 than 0 (p<0.025), but did not significantly differ between those with scores of 1 or 2. Regions with a higher SUVmax indicating active myocardial inflammation were mainly located in areas with LGE, where BMIPP and TL mismatches were evident in patients with CS.

Relationship between electrocardiographic findings and Cardiac Magnetic Resonance phenotypes in patients with Hypertrophic Cardiomyopathy

BackgroundQ waves and negative T waves are common electrocardiographic (ECG) abnormalities in patients with Hypertrophic Cardiomyopathy (HCM). Several studies correlated ECG findings with presence and extent of fibrosis and hypertrophy; however, their significance remains incompletely clarified. Our study aimed to explain the mechanism behind Q and negative T waves by comparing their positions on a 12-lead ECG with phenotypes observed at Late Gadolinium Enhancement (LGE) Cardiac Magnetic Resonance (CMR). Methods12-lead ECG and LGE-CMR were performed in 88 consecutive patients with HCM (42 SD 16years, 65 males). Using Delta Thickness ratio (DT ratio), and “global” and “parietal” LGE at CMR, the extent and distribution of myocardial hypertrophy and fibrosis were studied in correlation with ECG abnormalities. ResultsQ waves in different leads were not associated with “parietal” LGE score. Lateral Q waves correlated with an increased DT ratio Inferior Septum/Lateral wall (p=0.01). A similar correlation between inferior Q waves and an increased DT Ratio Anterior wall/Inferior wall was of borderline statistical significance (p=0.06). As expected, ECG signs of LV hypertrophy related to a raised Left Ventricular Mass Index (LVMI) (p<0.0001) and mean wall thickness (p=0.01). Depolarization disturbances, including negative T waves in lateral (p=0.044) and anterior (p=0.031) leads correlated with “parietal” LGE scores while QT dispersion (p=0.0001) was associated with “global” LGE score. ConclusionIn HCM patients, Q waves are generated by asymmetric hypertrophy rather than by myocardial fibrosis, while negative T waves result from local LGE distribution at CMR.

Extent of Late Gadolinium Enhancement on Cardiac Magnetic Resonance Imaging in Japanese Hypertrophic Cardiomyopathy Patients.

In addition to the presence of late gadolinium enhancement (LGE) on cardiovascular magnetic resonance (CMR), the extent of LGE is considered clinically important in hypertrophic cardiomyopathy (HCM). We evaluated the extent of LGE on CMR in a large series of Japanese HCM patients. CMR was performed in 317 HCM patients (147 male). The extent of LGE was scored as the sum of LGE-positive segments in a left ventricle (LV) 17-segment model. LGE was present in 246 patients (77.6%). LGE was detected in 3.5±3.1 segments on average. When the patients were divided according to maximum wall thickness (mild, <20 mm; moderate, 20-29 mm; severe, ≥30 mm), median LGE score increased as wall thickness increased (mild, 2 vs. moderate, 4 vs. severe, 5; P=0.000). When the patients were divided according to ejection fraction (EF) (reduced, <50%; low-normal, 50-65%; normal, >65%), median LGE score increased as EF decreased (reduced, 7 vs. low-normal, 4 vs. normal, 2; P=0.000). On multivariate analysis, reduced EF (OR, 0.947, P=0.015), pressure gradient <30 mmHg (OR, 0.359, P=0.000) and increased maximum wall thickness (OR, 1.236, P=0.000) were independent factors associated with extensive LGE. Progression of LGE was related to increased wall thickness, decreased contractility, and reduced intraventricular pressure gradient.

Late Gadolinium Enhancement on Cardiac MRI Correlates with QT Dynamicity Represented by QT/RR Relationship in Patients with Ventricular Arrhythmias

The distribution of late gadolinium enhancement (LGE) on the cardiac MRI (CMR) indicates myocardial fibrosis and provides information of possible reentry substrates. QT dynamicity reflecting repolarization abnormalities has gained attention as a potential prognostic predictive factor. To clarify the correlation between the LGE distribution on CMR and QT dynamicity represented by the QT/RR relationship. CMR and QT/RR analyses using Holter monitoring were performed in 34 patients (24 males, 60 ± 11 years) with ventricular tachycardia (VT) and/or ventricular fibrillation (VF). The LGE on CMR was scored using a 4-point score in 17 left ventricular segments. The sum of the LGE scores was calculated for each patient. The QT/RR slope and daytime/nighttime QT/RR ratio (day/night ratio) were calculated. The correlation between the slope or the day/night QT/RR ratio and late enhancement findings was analyzed. All patients were divided into 23 LGE positive (LGE(+)) and 11 LGE negative (LGE(-)) patients. The slopes of the QTe/RR and QTa /RR were significantly steeper in the LGE(+) than in LGE(-) patients (0.21 ± 0.03 vs 0.13 ± 0.02; P < 0.001, 0.19 ± 0.03 vs 0.13 ± 0.02; P < 0.001, respectively), and both slopes were significantly correlated with the total LGE scores (r = 0.83, P < 0.001; r = 0.71, P < 0.001, respectively). In the LGE(+) patients, the QTe day/night (1.37 ± 0.38 vs 0.91 ± 0.33; P = 0.002) and QTa day/night ratios (1.33 ± 0.26 vs 1.06 ± 0.30; P = 0.011) were significantly greater than those in the LGE(-) patients. The LGE distribution was closely related to the QT dynamicity, suggesting that a combination of these markers can be a powerful tool for understanding the background pathophysiology.

Restrictive right ventricular performance assessed by cardiac magnetic resonance after balloon valvuloplasty of critical pulmonary valve stenosis.

Little data are published about right ventricular diastolic performance in patients with critical pulmonary valve stenosis after balloon pulmonary valvuloplasty thus far. A total of 44 patients with isolated critical pulmonary valve stenosis who had undergone balloon valvuloplasty with haemodynamic recordings were enrolled to the study; 33 patients who came for follow-up underwent further imaging by echocardiography after 6 months and their right ventricular functional parameters were compared with 33 control patients of the same age and sex. Out of 33 patients, 21 underwent cardiac MRI with late gadolinium enhancement to assess the presence of right ventricular fibrosis. The right ventricular systolic pressure (p<0.0001) and right ventricular outflow tract gradient (p<0.0001) decreased acutely (p<0.0001) after balloon valvuloplasty. During follow-up, M-mode left ventricular end diastolic dimension (p<0.001) and end systolic dimension increased (p<0.001), whereas right ventricular end diastolic dimension decreased (p<0.001). Compared with controls, patients (n=33) had significantly reduced tricuspid annular Ea and higher E/Ea (p<0.001). Right ventricular systolic dysfunction was also suggested by reduced tricuspid annular systolic velocity (p<0.001). Late gadolinium enhancement was demonstrated in 13 out of 21 patients with restrictive physiology, which involves the anterior right ventricular outflow tract, anterior wall, and inferior wall. The right ventricular late gadolinium enhancement score correlated positively with age (r=0.7, p<0.001) and right ventricular mass index (r=0.52, p<0.001). The persistence of right ventricular diastolic dysfunction after relief of chronic pressure overload of critical pulmonary valve stenosis suggests that a factor - other than increase in afterload - is involved in this physiology. Fibrosis is the most likely factor responsible for persistence of restrictive physiology as documented by late gadolinium enhancement.